Published online Nov 27, 2021. doi: 10.4254/wjh.v13.i11.1727
Peer-review started: June 11, 2021
First decision: July 6, 2021
Revised: July 19, 2021
Accepted: September 16, 2021
Article in press: September 16, 2021
Published online: November 27, 2021
Core Tip: Cystic fibrosis(CF) liver disease is caused by abnormal cholangiocyte function, altered biliary secretion and abnormal innate immune response with abnormal response to endotoxins. CF liver disease can present with a wide variety of clinical features from a heterogenous liver on ultrasound, to life threatening gastrointestinal bleeds secondary to portal hypertension. Novel treatment strategies directly targeting the ion channel abnormality-cystic fibrosis transmembrane conductance regulator modulators are available and has significantly improved the clinical status and life expectancy of the cystic fibrosis patients.